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Granulomatous Flashcards

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1
Q

Subtypes of GA

A
  • Localised
  • Generalised
  • Subcutaneous
  • Perforating
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2
Q

Associated conditions of GA

A
  • Autoimmune thyroiditis
  • Hyperlipidaemia
  • ?Diabetes
  • No obvious relationship with malignancies, also think that some studies actually were interstitial granulomatous dermatitis as opposed to GA
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3
Q

Possible triggers of GA

A
  • Infection
    • Viral - HPV, HBV, EBV
    • Parasite - scabies
    • Bacterial - M tuberculosis, Borrelia
  • Trauma
    • Immunisation
    • Insect bites
    • Waxing
    • Red ink from tattoos –> perforating GA
  • Sunlight exposure
  • Drug induced - ADAPT3G:
    • Allopurinol
    • Diclofenac
    • Amlodipine
    • TNF alpha blockers, topiramate, thalidomide
    • Gold
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4
Q

Genetic mutation in GA

A

HLA-Bw35 in generalised

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5
Q

Histopathology of GA

A
  1. Necrobiotic palisading
    - ‘Blue granuloma’
    - Superficial and mid-dermis, separated by relatively normal tissue
    - Foci of necrobiosis, surrounded by histiocytes that may be palisading
    - Multi-nucleated giant cells
    - ‘Blue’: neutrophils and nuclear dust
    - Mucin - check with Alcian blue or colloidal iron stains
    - Reduction in elastic fibres
    - If subcutaneous –> larger areas of necrobiosis
    - If perforating –> transepithelial elimination, surface hyperkeratosis
  2. Interstitial
    - No formed areas of necrobiosis
    - Dermis looks ‘busy’ due to increased numbers of inflammatory cells
  3. Sarcoidal or tuberculoid
    - Increased dermal mucin or eosinophils differentiate it from sarcoidosis
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6
Q

Management of GA

A
  • Diagnosis - biopsy
  • Exclude differentials
    • Scrapings for fungal
    • Biopsy
  • Ascertain extent of disease
    • MRI if subcutaenous
  • Associations - diabetes (ask for signs and symptoms, no need to do bloods if don’t suspect), thyroid, lipids, malignancy hx
  • Treatment work up
    • Localised - steroid, tacrolimus, cryotherapy, intra-lesional steroid injection, nitrous oxide
    • Generalised - PUVA, NBUVB, retinoids, dapsone, MTX, plaquenil, cyclosporin, Humira
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7
Q

Sarcoidosis epidemiology

A

Epidemiology

  • Bi-modal distribution - 25-35 and 45-44
  • Non-Caucasian
  • Seasonal: new cases in winter and spring
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8
Q

Sarcoidosis pathophysiology

A
  • Environmental
    • Seasons
    • Occupation - i.e. firefighters
    • Exposures to moulds, tree pollens and insecticides
  • Infectious agents
    • Viral: EBV
    • Bacterial: P acnes, TB, Rickettsia, Chlamydia, Lyme disease
  • Medications
    • TNF alpha blockers
    • immune checkpoint inhibitors
    • targeted kinase inhibitors
  • Genetics
    • Family history
    • HLA-DR alleles: DRB1*03, 11 12, 14, 15 –> confer risk, and HLA-B8/DR3 associated with Lofgren, Afticans HLA-DQB1
    • Gene TNF associated with Lofgren, and IL23R risk for CD and Sarcoidosis
  • Associated conditions
    • Lymphoproliferative: particularly NHL
    • Autoimmune: Sjogren, systemic sclerosis, rheumatoid arthritis, thyroid
    • Abnormal accumulation of amyloid
  • Cellular change:
    • antigen incites macrophages to accumulate and become multinucleared giant cell –> inciting a Th1 response, and CD4 T cells at the centre of the granuloma (interferon gamma and IL-2)
    • There is some TH2 response which makes fibrosis, so there are CD8 T cells at the periphery
    • When there is T cell accumulation this means less T cells actually working resulting in anergy
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9
Q

Maculopapular sarcoidosis

A

Yellow-brown or red-brown without clinically evident epidermal changes
Face
Transient
Resolve spontaneously or with treatment, don’t scar
Associations: more favourable prognosis, acute forms - hilar lymphadenopathy, EN, acute uveitis, lymphadenopathy
Subtype of this type: extensor linear papules (knees) - easily overlooked

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10
Q

Sarcoidosis - nodules and plaques

A

Face, scalp, back, buttocks and extremities
When resolve can leave permanent scarring
Associated with chronic forms - pulmonary fibrosis, lymphadenopathy, chronic uveitis
Not associated with bone or sarcoidosis of the upper respiratory tract

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11
Q

Scar sarcoidosis

A

Can occur decades later. Foreign material in scar acts as antigenic stimulus for induction of granulomas
Old scars become infiltrated and erythematous and contain sarcoid granulomas histopathologically
Frequently on the knees
Associated with long-lasting pulmonary and mediastinal involvement, uveitis, peripheral lymphadenopathy, bony cysts and parotid infiltration

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12
Q

Subcutaneous sarcoidosis

A

Limited to subcutaneous tissue, minimal dermal involvement
White women, 5th - 6th decades of life
Painless
Sssociated with stage 1 changes on chest x0ray and less than 2 years activity of systemic sarcoidosis
Darier-Roussy is a type of S/C sarcoidosis: painless firm subcutaneous nodules or plaques on the trunk and extremities, often associated with systemic sarcoidosis

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13
Q

Lofgren’s

A 
Hilar adenopathy, fevers, arthritis and EN
Resolve spontaneously over 1-2 years
Lymphadenopathy
O
Fever
Generalised malaise
Arthritis
Erythema nodosum
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14
Q

Lupus pernio

A

More common in black women
Papulonodules and plaques, on nose, cheeks and ears and have violaceous colour. Disfiguring. Chronic.
Associated with chronic sarcoidosis of the lungs
Associated with URTI, pulmonary fibrosis, chronic uveitis and bony cysts - terminal phalanges

Discrete variant: angiolupoid - prominent large, telangiectatic venules. Single raised plaque on bridge of nose, central face, ears or scalp

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15
Q

Sarcoidosis - LEGS

A

Ulcerative: papulonodular or atrophic lesions on lower legs and heals with scarring
NLD like
Morphoea like - indurated and atrophic plaques, located on thighs of black women
EN - generally good prognosis, runs benign and self-limited course.

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16
Q

Non-specific Sarcoid changes

A

Hair changes: scarring alopecia, absent scale
Nail changes: subungual hyperkeratosis, clubbing, onycholysis, splinter haemorrhages, thinning, pitting, paronychia, pterygium, trachyonychia
Oral: infrequent, diffuse sub-mucous thickening or firm nodule in buccal mucosa
Genital: testicular or epidydmal masses

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17
Q

Heerfordt syndrome:

A

uveoparotid fever - parotidomegaly, uveitis, fever, cranial nerve palsies

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18
Q

Systemic manifestations of sarcoidosis

A
  • Lung - 90%, upper lungs, fibrosis, honeycombing.
    • Ranges from alveolitis to granulomatous infiltration of the alveoli, blood vessels, bronchioles, pleura and fibrous septa.
    • End stage: bronchiolectasis and honeycombing.
    • 90% have hilar and/or paratracheal lymphadenopathy
  • Lymphatics - 30-40%
  • Ocular - 25%, uveitis, retinal vasculitis, conjunctivitis
  • Neurosarcoidosis - prediliction for basal meninges –> cranial nerve involvement
  • URT - sinusitis, nasal congestion, stridor, parotiditis, parotid enlargement
  • Endocrine - pituitary or thyroid dysfunction, hypercalcaemia
  • Cardiac - arrhythmias, cardiomegaly, sudden death
  • Liver/spleen
  • Bone marrow - 50%, lymphopenia or leukopenia, eosinophilia, hypergammaglobulinaemia –> ?risk of lymphoma development
  • Bones - lysis
  • MSK - arthritis, weakness
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19
Q

Sarcoidosis histology

A
  • Epidermis usually normal, may have lichenoid or transepithelial elimination
  • Naked granuloma –> non-caseating epithelioid granuloma. Granulomas are Langhans giant cells. Pauci-inflammatory.
    • Superficial and deep
    • Central caseation usually absent
    • Fibrinoid deposition may be seen in up to 10%
    • Giant cells are usually of Langhans type - nuclei arranged in a peripheral arc or a circular fashion
  • Predominantly in dermis, if subcutaneous will just be in S/C fat
  • Particular things to look for - though not specific to sarcoidosis:
    • Asteroid bodies - stellate eosinophils in centre, trapped collagen, in giant cells. Represent engulfed collagen
    • Schaumman bodies - basophilic, round/concentric lamellar structures impregnated with calcium and iron, in giant cells, represent degenerating lysosomes
    • Crystalline bodies
  • Up to 20% contain polarizable material
  • Vulval: may have transepidermal elimination
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20
Q

Histological differential diagnoses for sarcoidosis

A
  • Lupus vulgaris
  • Tuberculous leprosy
  • Rosacea granulomas
  • Syphilis
  • Crohns
  • Orofacial granulomatosis
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21
Q

Investigations for sarcoidosis

A
  • Diagnosis
    • FBC, UEC, ACE levels
    • Biopsy
  • Exclude differentials
    • Various - Tuberculin skin tests, syphilis screen, etc
  • Ascertain extent of disease
    • Opthal review
    • CXR and pulmonary function test (decreased forced vital capacity)
    • CMP - hypercalcaemia
    • LFT
    • ECG
  • Associated diseases
    • EPG/IEPG, FBC and blood smear
    • Autoimmune - ANA and ENA
  • Treatment work up
    • Depends - vitamin D, bone mineral density
  • Horrible complications
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22
Q

Treatment for cutaneous sarcoidosis

A 
Topical or IL steroids
Topical calcineurin inhibitors
Oral tetracyclines
Plaquenil or chloroquine
MTX
Systemic steroids
Thalidomide
TNF alpha inhibitors
Apremilast and pentoxifylline
Leflunomide
Intralesional chloroquine
Allopurinol
Isotretinoin
JAK inhibitor: tofacitinib
PUVA
MMF
Pulsed dy or CO2 laser
PDT
Surgical excision
23
Q

Actinic granuloma pathophysiology

A
  • believed to be inflammation precipitated by actinic damage

- possibly weakly antigenic determinant on altered elastotic fibres resulting in T lymphocyte helper subset (CD4)

24
Q

Actinic granuloma clinical

A
  • Medium sized to large annular plaques with a raised erythematous border
  • Slightly atrophic and hypopigmented centre
  • Sun exposed sites: face, neck, upper trunk, arms
  • No alopecia, no yellow, no telangiectasias
  • Lasts for many years, may undergo spontaneous remission
25
Q

Histology of actinic granuloma

A
  • Non palisaading granulomatous infiltrates of histiocytes, foreign body type multinucleated giant cells (haphazardly arranged nuclei) and lymphocytes in mid to upper dermis
  • Absence of collagen changes or mucin
  • Van Gieson stain will show complete absence of elastic fibres in the areas affected
  • stain for acid fast bacilli and fungal organisms to exclude infectious granulomatous
26
Q

Ddx of actinic granuloma

A
  • Clinical
    • all the annulars
    • EAC
    • annular LP
    • secondary syphilis
    • tinea corporis
    • other granulomatous infections
  • Histological
    • granulomatous slack skin
    • mid-dermal elastolysis
    • anetoderma
    • acquired cutis laxa
27
Q

Treatment of actinic granuloma

A
  • responds poorly or inconsistently to therapy
  • topical or intralesional steroids
  • PUVA
  • case reports: dapsone, systemic retinoids, cyclosporine, chloroquine
  • Procedural: excision
  • Ineffective: cryotherapy, cauterisation, methotrexate
28
Q

NLD Epidemiology

A
  • young to middle aged
  • F>M
  • Diabetics: average age 25
  • Non-diabetics: 46 years average age
  • Obviously association with diabetes, however no proven connection with glycaemic control, however do have higher rate of diabetes related complications
29
Q

NLD pathogenesis

A
  • Postulated to be microangiopathic vessel changes leading to collagen degeneration and subsequent dermal inflammation
  • Also thought venous hypertension, venous insufficiency and hyperlipidaemia incite inflammation
  • Some believe to be primarily disease of collagen
30
Q

NLD Clinical features

A
  • Yellow-brown atrophic, telangiectactic plaques with an elevated violaceous rim in the pre-tibial region
  • Multiple and bilateral
  • 1/3 –> ulceration
  • Reports of Koebnerisation
  • Decreased sensation to pinprick and fine touch, hypohidrosis and partial alopecia
  • Decreased S100 staining ? neural involvement
  • Risk: SCC development
  • Dermoscopy: comma shaped vessels and irregular pattern of arborizing vessels, whitish areas correspond to degenerated collagen and yellow to orange to granulomatous inflammation
31
Q

NLD Histology

A
  • Epidermis normal or atrophic
  • Diffuse palisaded and interstitial granulomatous dermatitis - involves entire dermis and extends into the subcutaneous fat septae
  • Necrobiosis - ‘red’, may have flame figures
  • Giant cells
  • ‘Layer’ or ‘lasagne’ –> takes up whole dermis
  • Areas of sclerosis - focal loss of elastic tissue may be seen
  • Superficial and deep perivascular infiltrate that is predominantly lymphocytic, but can have plasma cells
32
Q

NLD compared to GA on histopath

A
  • prominent endothelial cell swelling, fibrosis and hyalinization –> blood wall thickening
  • more collagenolysis
  • ‘red’ granuloma
  • Minimal mucin, GA has more mucin
33
Q

NLD differentials

A
  • Clinically:
    • GA
    • Necrobiotic xanthogranuloma
    • Sarcoidosis
    • Diabetic dermopathy
    • Lipodermatosclerosis
    • Panniculitides
    • Granulomatous infections
    • Morphoea
    • Lichen sclerosus
    • Sclerosing lipogranuloma
  • Histologically:
    • GA
    • NXG
34
Q

NLD Treatment

A
  • Investigate and treat diabetes
  • Topical/intralesional steroids, or topical tacrolimus
  • Ulcerations: topical GM-CSF or bovine collagen, or tretinoin
  • Avoid trauma and local care of ulcers
  • Anecdotal reports: pentoxifylline, stanozolol, inositol niacinate, etc
  • No evidence for aspirin
  • Systemics: anti-malarials, niacinamide, mycophenolate, doxycycline, colchicine, methotrexate, thalidomide, tnf-alphas, cyclosporin, systemic steroids
  • Last case reports:
    • PUVA
    • fractional CO2
    • PDT
  • Surgical: excision –> need to do to deep fascia
35
Q

Crohn’s categories

A
  • Specific lesions
  • Non-specific or reactive
  • Nutritional skin changes
  • Treatment related side effects
36
Q

Crohn’s pathogenesis

A
  • Genetic abnormalities –> exaggerated T-cell response to certain commensal enteric bacteria and defective microbial clearance
  • Patients with variants in TRAF3IP2 (a psoriasis susceptibility gene) may have a greater risk of cutaneous involvement
  • Predominantly Th1 and Th17 mediated
37
Q

Genital crohns

A
  • Genital
    • 2/3 kids and 1/2 adults
    • Labial, penile or scrotal erythema
    • Linear or knife like ulcerations that are within body folds or oral mucosa
    • Peri-anal: sinus tracts, fissures, ulcers, vegetating plaques, skin tags secondary to oedema
38
Q

Non-genital Crohns

A
  • Oral
    • 5-20% of patients
    • cobblestoning of the buccal mucosa, gingival hyperplasia, aphthae like ulcers, linear knife-like ulcerations, pyostomatitis vegetans, angular cheilitis
    • 90% of these lesions have granulomas
  • Non-genital
    • dusky erythematous plaques, often followed by development of ulceration with undermined edges, draiing sinuses and fistulas and scarring
    • Occur lower extremities, soles, trunk, epper extremities, etc
    • Can have EN, PAN, EM, finger clubbing, vasculitis, EBA, vitiligo, palmar erythema
39
Q

Crohn’s histo

A
  • Nodular caseating, epithelioid tubercles with surrounding lymphocytes –> can extend into subcutaneous fat
  • Scattered multinucleated Langhans type giant cells
  • Sparse peri-vascular lymphohistiocytic infiltrate
  • Clue: lichenoid + granulomatous inflammation + granulomatous peri-vasculitis
40
Q

Crohn’s ddx

A
  • Clinically
    • Sarcoidosis
    • Mycobacterial infections
    • Deep fungal infections
    • Foreign body reactions
    • Ulcerated: PG
    • Genital swelling: granuloma inguinale, schistosomiasis, HS, chronic lymphoedema
  • Histo:
    • Granulomatous - lupus vulgaris, foreign body, infectious, etc
41
Q

Crohn’s Rx

A
  • severity not related to intestinal disease
  • Local: topical, IL steroids or topical calcineurin inhbiitors
  • Systemic: oral metronidazole 250 mg TDS for 4 months
  • Extensive: steroids, sulfasalazine, azathioprine, 6-mercaptopurine, TNF alpha blockers, thalidomide
  • Surgical excision
42
Q

IGD and PNGD pathogenesis

A
  • initiating insult (trauma) –> immune complex deposition on or around the walls of the small dermal blood vessels –> subacute or chronic neutrophil rich small vessel vasculitis
  • this impairs blood flow in a smouldering fashion (not abrupt blood flow stop) –> resulting in degenerating collagen
  • that degenerated collagen results in an immune response –> palisading lymphohystiocytic infiltrate
  • Its essentially a granulomatous response to degenerating collagen
43
Q

Clinical PNGD

A
  • skin coloured to erythematous papules symmetrically on extensor surfaces
  • central umbilication with crusts or perforation
  • associated with RA, SLE, systemic vasculitis
44
Q

Clinical IGD

A
  • erythematous plaques with an annular configuration or linear cords (rope sign)
    • favour lateral trunk, azillae, buttocks, medial thighs and groin
    • women with RA, seronegative arthritis or polyarthralgies, autoimmune thyroiditis
45
Q

Histology PNGD

A
  • early: pandermal infiltrate of neutrophils, sometimes with focal leukocytoclastic vasculitis
  • Fully developed: zones of basophilic degenerated collagen, surrounded by palisades of histiocytes, neutrophils and nuclear debrist
  • Incomplete early: focal basophilic collagen necrosis and histiocytes without orderly palisading + neutrophils
  • Late: well-developed palisading granulomas
  • Mucin is rare
46
Q

HIstology IGD

A

Rosettes of palisading histiocytes surrounding tiny, discrete foci of degenerated collagen with a dense, bottom heavy dermal interstitial infiltrate that contains variable numbers of neutrophils and eosinophils

47
Q

IGD and PNGD treatment

A
  • no specific therapy

- Steroids, dapsone, plaquenil

48
Q

Childhood sarcoid

A

Rare
Triad of arthritis, uveitis and cutaneous lesions with constitutional sx
Peripheral LN is frequent
If being considered, need to exclude Blau syndrome

49
Q

List all types of cutaneous sarcoid

A 
Maculopapular, papular
Nodules and plaques
Scar
Subcutaneous - Darier Roussy subtype
Lofgrens
Lupus pernio
Hypopigmented
Lichenoid
Ulcerative, NLD, morphoea, EN
Psoriasiform
Verrucous
Ichthyosiform
Erythrodermic
Livedo
Rare: DLE, LS, lipodermatosclerosis, cellulitis, breast carcinoma, follicular, photoinduced
50
Q

Hair changes in sarcoidosis

A

Scarring alopecia

Absent scale

51
Q

Nail changes in sarcoidosis

A

subungual hyperkeratosis, clubbing, onycholysis, splinter haemorrhages, thinning, pitting, paronychia, pterygium, trachyonychia

52
Q

Genital changes in sarcoidosis

A

testicular or epidydmal masses

53
Q

IGD drug causes

A

Calcium channel blockers
Statins
TNF alpha inhibitors

Derm (57 decks)

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